Neonatalis lupus erythematosus: irodalmi összefoglalás egy esetbemutatás kapcsán = Neonatal lupus erythematosus: case report and review of the literature

A neonatalis lupus erythematosus (NLE) az ujszulottek első nehany honapjanak megbetegedese, melyet az edesanya autoimmun betegsegenek (SLE, Sjogren, rheumatoid arthritis stb.) kapcsan termelődő, placentan atjuto anti-SSA- es anti-SSB-ellenanyagok okoznak. Ritka megbetegedes. Tunetei erinthetik a bőrt (jellegzetes annularis, erythemas plakkok), a szivet, a majat es a verkepzest. Legsulyosabb kovetkezmenye a 20–30%-os mortalitasert elsősorban felelős III. foku AV-blokk, mely maradando karosodas, s pacemaker beulteteset teszi szuksegesse. A tunetek altalaban kezeles nelkul megszűnnek az anyai ellenanyagok kiurulesevel parhuzamosan a csecsemő 6–9 honapos korara. Esetunkben a tipikus bőrtunetek az egesz testet erintettek, mar szuleteskor fennalltak, de szivblokk nem alakult ki. A diagnozist jelentősen nehezitette, hogy az edesanya betegsege nem volt ismert a szules idejen. A jelentős thrombocytopenia, progredialo bőrtunetek es emelkedett majfunkcios ertekek miatt szisztemas szteroid adasara kenyszerultunk. | Neonatal lupus erythematosus (NLE) is a disease of the first few months of infancy. It is caused by anti-SSA and anti-SSB antibodies, which are products of maternal autoimmune disorders (SLE, Sjogren, rheumatoid arthritis) and can be passively transported across the placenta. The prevalence of NLE is low. The major clinical findings are cutaneous (typical annular erythematous plaques), cardiac, hepatic and hematologic alterations. Its most severe consequence is the third-degree heart block, which is irreversible, requires pacemaker-implantation and responsible for the 20–30% mortality rate. Symptoms usually resolve spontaneously at age of 6–9 months in association with disappearance of maternal antibodies from the infant’s serum. In our case the typical cutaneous manifestations covered virtually the whole body, were present at birth, however, no conduction defects developed. The fact that the mother’s sickness was not known at birth made it difficult to give the diagnosis. The significant thrombocytopaenia, progressive skin-changes and the elevated liver function tests demanded systemic steroid treatment.